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Paeds: Acute Presentation > Congenital and acquired cardiac disease > Flashcards

Congenital and acquired cardiac disease Flashcards

1
Q

What are 9 of the most common congenital heart lesions in children?

A
  1. Ventricular septal defects
  2. Persistent arterial duct
  3. Atrial septal defect
  4. Tetralogy of Fallot
  5. Transposition of the great arteries
  6. Atrioventricular septal defect (complete)
  7. Pulmonary stenosis
  8. Aortic stenosis
  9. Coarctation of the aorta
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2
Q

What are 3 left-to-right shunts?

A
  1. Ventricular septal defect
  2. Atrial septal defect
  3. Persistent ductus arteriosus
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3
Q

What is the one word to describe the presentation of left-to-right shunts?

A

breathless

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4
Q

What are 2 causes of right-to-left shunts?

A
  1. Tetralogy of Fallot
  2. Transposition of the great arteries
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5
Q

What is a one word to describe the presentation of right-to-left shunts?

A

blue

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6
Q

What is the key example of a common mixing (breathless and blue) heart defect?

A

atrioventricular septal defect (complete)

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7
Q

What are 2 examples of outflow obstruction in a well child?

A
  1. Pulmonary stenosis
  2. Aortic stenosis
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8
Q

What is the usual presentation of outflow obstruction in a well child?

A

asymptomatic with a murmur

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9
Q

What is a key example of outflow obstruction in a sick neonate?

A

coarctation of the aorta

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10
Q

What is the usual presentation of outflow obstruction in a sick neonate?

A

collapsed with shock

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11
Q

What can cause congenital heart disease?

A

genetics: whole chromosomes, point mutations, microdeletions

Polygenic abnormalities

Small number related to external teratogens

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12
Q

What are 5 environmental causes of congenital heart disease?

A
  1. Rubella infection
  2. Systemic lupus erythematosus
  3. Diabetes mellitus
  4. Warfarin therapy
  5. Fetal alcohol syndrome
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13
Q

What are 2 types of congenital heart disease that maternal rubella infection can cause?

A
  1. Peripheral pulmonary stenosis
  2. PDA
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14
Q

What heart defect can maternal SLE cause?

A

complete heart block

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15
Q

What are 2 heart defects that maternal warfarin therapy can cause?

A
  1. Pulmonary valve stenosis
  2. PDA
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16
Q

What are 3 congenital heart problems caused by fetal alcohol syndrome?

A
  1. ASD
  2. VSD
  3. Tetralogy of Fallot
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17
Q

What are 7 chromosomal abnormalities which can cause congenital heart problems?

A
  1. Down syndrome (trisomy 21)
  2. Edwards syndrome (trisomy 18)
  3. Patau syndrome (trisomy 13)
  4. Turner syndrome (45,XO)
  5. Chromosome 22q11.2 deletion
  6. Williams syndrome (7q11.23 microdeletion)
  7. Noonan syndrome (PTPN11 mutation and others)
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18
Q

What are the 2 commonest cardiac abnormalities caused by Down syndrome?

A
  1. Atrioventricular septal defect
  2. VSD
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19
Q

What are 2 of the commonest cardiac abnormalities caused by Turner syndrome?

A
  1. Aortic valve stenosis
  2. Coarctation of the aorta
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20
Q

What is the circulation of the fetus in utero?

A

left atrial pressure low as little blood returns from lungs

pressure in right higher than left as receives all sytemic venous return including blood from placenta

flap valve of foramen ovale held open, blood flows across atrial septum into left atrium, then into left ventricle, which pumps it to the upper body

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21
Q

What happens to the circulation when the fetus is born?

A
  • with first breaths, resistance to pulmonary blood flow falls and volume of blood flowing through lungs increases 6 fold
  • results in rise in left atrial pressure
  • volume of blood returning to right atrium falls as placenta excluded from circulation
  • causes flap valve of foramen ovale to close
  • ductus arteriosus between pulmonary artery to aorta usually closes within first hours or days
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22
Q

When can the closure of the ductus arteriosus be negative?

A

some babies with congenital heart lesions rely on blood flow through the duct (duct-dependent circulation) so clinical condition will deteriorate dramatically when duct closes (1-2 days of age usually)

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23
Q

When does the ductus arteriosus normally close?

A

1-2 days of age

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24
Q

What are 5 ways that congenital heart disease may present?

A
  1. Antenatal cardiac ultrasound
  2. Detection of heart murmur
  3. Heart failure
  4. Shock
  5. Cyanosis
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25
Q

What is done if an abnormality is detected at the fetal anomaly scan between 18-20 weeks’ gestation?

A

detailed fetal echocardiography performed by paediatric cardiologist

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26
Q

What is the likely management of a fetus with a duct-dependent lesion of the heart detected antenatally?

A

need treatment within the first 2 days of life; may be offered delivery at or close to the cardiac centre

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27
Q

In addition to when a heart abnormality is detected on routine fetal anomaly scans, what are 3 other situations when the detailed fetal echo is performed?

A
  1. Down syndrome suspected
  2. Parents have had previous child with heart disease
  3. Mother has congenital heart disease
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28
Q

What is the most common presentation of congenital heart disease?

A

heart murmur

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29
Q

What proportion of children have an innocent murmur - i.e. a normal heart?

A

30% have one at some time

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30
Q

What are 7 hallmarks of an innocent ejection murmur?

A
  1. Asymptomaic
  2. Soft blowing murmur
  3. Systolic murmur only, not diastolic
  4. Left sternal edge
  5. Normal heart sounds with no added sounds
  6. No parasternal thrill
  7. No radiation
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31
Q

When are innocent or flow murmurs often heard in children? What should be done due to this?

A

during febrile illness or anaemia - examine child when other illnesses have been corrected

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32
Q

What should you do if you think you detect a significant murmur in a child or are unsure?

A

child should be seen by experienced paediatrician to decide abuot referral to paediatric cardiologist for echocardiography

CXR and ECG may help

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33
Q

Why might conditions such as ventricular septal defect or PDA only become apparent at several weeks of age?

A

pulmonary vascular resistance still high in newborns

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34
Q

What are 4 symptoms of heart failure?

A
  1. breathlessness
  2. sweating
  3. poor feeding
  4. recurrent chest infections
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35
Q

What are 7 signs of heart failure?

A
  • Poor weight gain or faltering growth
  • Tachypnoea
  • Tachycardia
  • Heart murmur, gallop rhythm
  • Enlarged heart
  • Hepatomegaly
  • Cool peripheries.
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36
Q

What is the usual cause of heart failure in the first week of life?

A

left heart obstruction e.g. coarctation of the aorta

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37
Q

Which can closure of the ductus arteriosus lead to severe consequences in coarctation of the aorta?

A

if anything causes a severe obstructive lesion, arterial perfusion may be predominantly by right to left flow of blod via arterial duct - duct-dependent systemic circulation

closure can rapidly lead to severe acidosis, collapse and death unless ductal patency restored

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38
Q

What are 4 causes of heart failure in neonates?

A
  1. Hypoplastic left heart syndrome
  2. Critical aortic valve stenosis
  3. Severe coarctation of the aorta
  4. Interruption of the aortic arch
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39
Q

What is the typical cause of heart failure in neonates?

A

obstructed (duct dependent) systemic circulation

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40
Q

What is the typical cause of heart failure in infants?

A

high pulmonary blood flow

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41
Q

What are 3 examples of causes of heart failure in infants?

A
  1. Ventricular septal defect
  2. Atrioventricular septal defect
  3. Large persistent ductus arteriosus
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42
Q

What is usually the cause of heart failure in older children and adolescents?

A

right or left heart failure

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43
Q

What are 3 examples of causes of heart failure in older children and adolescents?

A
  1. Eisenmenger syndrome (right heart failure only)
  2. Rheumatic heart disease
  3. Cardiomyopathy
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44
Q

What is the key to early survival in neonates with a duct-dependent circulation?

A

maintaining ductal patency

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45
Q

Why is progressive heart failure most likely due to a left-to-right shunt after the first week of life?

A

as pulmonary vascular resistance falls, there is a progressive increase in left-to-right shunting and increasing pulmonary blood flow

this causes pulmonary oedema and breathlessness

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46
Q

In the case of heart failure due to a left-to-right shut, what is the likely natural time course?

A

symptoms of heart failure will icrease up to age 3 months but may subsequently improve as pulmonary vascular resistance rises in response to the left-to-right shunt

if untreated children will develop Eisenmenger syndrome - raised pulmonary vascular resistance from chronically raised pulmonary arterial pressure and flow

shunt now from right to left and teenager blue

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47
Q

What is Eisenmenger syndrome?

A

irreversibly raised pulmonary vascular resistance due to chronically raised pulmonary arterial pressure and flow

shunt goes from left-to-right to right-to-left and child becomes blue

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48
Q

What is the only management option for Eisenmenger syndrome?

A

heart-lung transplant (medication available to palliate)

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49
Q

What can cause peripheral cyanosis?

A

in hands and feet - may occur if child cold or unwell from any cause or with polycythaemia

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50
Q

What causes central cyanosis?

A

slate blue tongue: due to fall in arterial blood oxygen tension

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51
Q

When can central cyanosis be recognised clinically?

A

if concentration of reduced Hb is >50g/L so less pronounced if child is anaemic

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52
Q

What is the first investigation to perform in a child with cyanosis?

A

check with pulse oximeter that infant’s sats are ≥94%

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53
Q

What is nearly always the cause of persistent cyanosis in an otherwise well infant?

A

structural heart disease

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54
Q

What are 5 causes of cyanosis in a newborn infant with respiratory distress?

A
  1. Cardiac disorders - cyanotic congenital heart disease
  2. Respiratory disorders e.g. respiratory distress syndrome (surfactant deficiency), meconium aspiration, pulmonary hypoplasia
  3. Persistent pulmonary hypertension of the newborn - failure of pulmonary vascular resistance to fall after birth
  4. Infection - sepsis from group B streptococcus and others
  5. Inborn error of metabolism - metabolic acidosis and shock
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55
Q

What respiratory rate defines respiratory distress in a newborn?

A

>60 breaths/min

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56
Q

What are 3 investigations to perofrm if congenital heart disease is suspected?

A
  1. CXR
  2. ECG
  3. Echo combined with Doppler untrasound
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57
Q

What is the benefit of ECG and CXR in suspected congenital heart disease?

A

rarely diagnostic but helpful in establishing there is an abnormality of CVS and as baseline for assessing future changes

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58
Q

How can you get a diagnostic echo with Doppler ultrasound for heart disease performed even if no paediatric cardiologist is available locally?

A

specialist echo opinion may be available via telemedicine, otherwise transfer to cardiac centre will be necessary

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59
Q

What are 5 situations when a specialist opinion is needed for cardiac disease?

A
  1. Haemodynamically unstable
  2. Heart failure
  3. Cyanosis
  4. Oxygen sats <94 due to heart disease
  5. Reduced volume pulses
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60
Q

What are 4 important features of use in ECG in children?

A
  1. Arrhythmias
  2. Superior QRS axis (negative deflection in AVF)
  3. Right ventricular hypertrophy (upright T wave in V1 over 1 month of age)
  4. Left ventricular strain (inverted T wave in V6)
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61
Q

What are 3 pitfalss of ECG in children?

A
  1. P-wave morphology is rarely helpful in children
  2. Partial right bundle branch block – most are normal children, although it is common in ASD
  3. Sinus arrhythmia is a normal finding
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62
Q

What drug can be given to maintain ductus arteriosus patency in duct-dependent cyanosis?

A

prostaglandins

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63
Q

How can heart disease cause shock?

A

when duct closes in severe left-heart obstruction

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64
Q

What are the 2 types of atrial septal defect?

A
  1. Secundum ASD
  2. Partial atrioventricular septal defect (partial AVSD or primum ASD)
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65
Q

What is the commonest type of ASD?

A

secundum ASD

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66
Q

What is secumdum AVSD?

A

defect in the centre of the atrial septum involving the foramen ovale

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67
Q

What is partial AVSD?

A

defect of the atrioventricular septum, characterised by:

  • interartrial communication between the bottom end ofthe atrial septum and the atrioventricular valves (primum ASD)
  • abnormal atrioventricular valves, with a left atrioventricular valve whcih has three leaflets and tends to leak (regurgitant valve)
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68
Q

What are 3 forms of symptoms of ASD?

A
  1. None (commonly)
  2. Recurrent chest infections/ wheeze
  3. Arrhythmias (fourth decade onwards)
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69
Q

What are 3 physical signs of ASD?

A
  1. Ejection systolic murmur at upper left sternal edge (both)
  2. Fixed and widely split second heart sound (often hard to hear) (both)
  3. Partial AVSD: apical pansystolic murmur
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70
Q

What causes the ejection systolic murmur in ASD?

A

increased flow across the pulmonary valve because of the left-to-right shunt

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71
Q

What causes the fixed and widely split second heart sound in ASD?

A

right ventricular stroke volume being equal in both inspiration and expiration

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72
Q

What causes the apical pansystolic murmur in partial AVSD?

A

from atrioventricular valve regurgitation

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73
Q

What will a chest radiography show in ASD? 3 things

A
  1. Cardiomegaly
  2. Enlarged pulmonary arteries
  3. Increased pulmonary vascular markings
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74
Q

What are 2 things that secundum ASD can show on ECG?

A
  1. Partial right bundle branch block is common
  2. Right axis deviation due to right ventricular enlargement
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75
Q

What are may be seen on ECG in partial AVSD and why?

A

superior QRS axis due to defect of the middle part of heart where AV node is

displaced node conducts to the ventricles superiorly, giving abnormal axis

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76
Q

What is the mainstay of diagnosis for ASD?

A

Echo - will delineate the anatomy

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77
Q

Where can a ventricular septal defect occur?

A

anywhere in the ventricular septum, perimembranous (adjacent to tricuspid valve) or muscular (completely surrounded by muscle)

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78
Q

What are the two groups that VSDs can be divided into?

A
  1. Small VSDs - smaller than aortic valve in diameter, up to 3mm
  2. Large VSDs - same size or bigger than aortic valve
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79
Q

What are the symptoms of small VSDs?

A

asymptomatic

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80
Q

What are 2 physical signs of VSDs?

A
  1. Loud pansystolic murmur at lower left sternal edge (loud murmur implies smaller defect)
  2. Quiet pulmonary second sound (P2)
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81
Q

How does the volume of a murmur correlate with the size of the defect?

A

louder sound = smaller defect

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82
Q

Which will CXR and ECG show for small VSDs?

A

normal

83
Q

What will echocardiography show for small VSDs?

A

precise anatomy of defect; can assess haemodynamic effects using Doppler echo

no pulmonary hypertension

84
Q

What is the management of small VSDs?

A
  • will close spontaneously
    • disappearance of murmur with normal ECG on follow up and by normal echo
  • while present, prevention of bacterial endocarditis by maintaining good dental hygiene
85
Q

What are 2 symptoms of large VSDs?

A
  1. Heart failure with breathlessness and faltering growth after 1 week old
  2. Recurrent chest infections
86
Q

What are 5 physical signs of large VSDs?

A
  1. Tachypnoea, tachycardia and enlarged liver from heart failure
  2. Active precordium
  3. Soft pansystolic murmur or no murmur (implying large defect)
  4. Apical mid-diastolic murmur
  5. Loud pulmonary second sound (P2)
87
Q

What causes the apical mid-diastolic murmur from large VSDs?

A

increased flow across the mitral valve after blood has circulated through the lungs

88
Q

What causes the loud pulmonary second sound in VSD?

A

raised pulmonary arterial pressure

89
Q

What will be 4 things on the CXR in large VSDs?

A
  1. Cardiomegaly
  2. Enlarged pulmonary arteries
  3. Increased pulmonary vascular markings
  4. Pulmonary oedema
90
Q

What will the ECG show in large VSDs?

A

biventricular hypertrophy by 2 months of age

91
Q

What will echo show in large VSD?

A

demonstrates anatomy of defect, haemodynamic effects and pulmonary hypertension (due to high flow)

92
Q

What is the management of large VSD? 3 aspects

A
  • drug therapy for heart failure with diuretics + captopril
  • additional calorie input required
  • Surgery at 3-6 months to prevent Eisenmenger syndrome and manage heart failure
93
Q

When should surgery usually be performed to repair large VSD?

A

3-6 months of age

94
Q

What is the definition of persistent ductus arteriosus?

A

has failed to close by 1 month after expected date of delivery due to defect in constrictor mechanisms of duct

can occur in preterm infant due to prematurity

95
Q

What happens in persistent ductus arteriosus?

A

flow of blood across PDA is from aorta to pulmonary artery (left to right shunt) following fall in pulmonary vacular resistance after birth

96
Q

What are 3 physical signs of persistent ductus arteriosus?

A
  1. Continuous murmur beneath left clavicle
  2. Murmur continues into diastole because pressure in the pulmonary artery is lower than that in the aorta throughout the cardiac cycle
  3. Pulse pressure increased - collapsing or bounding pulse
97
Q

What are symptoms of PDA?

A

unusual to get symptoms but can get heart failure if the duct is large and there’s increased pulmonary blood flow + pulmonary hypertension

98
Q

What may ECG and CXR show in PDA?

A

usually normal, but if large and symptomatic, features are indistinguishable from those seen in large VSD

99
Q

What is the management of PDA?

A

close with coil or occlusion device introduced via cardiac catheter at 1 year of age

Occasionally surgical ligation required

100
Q

At what age is surgery to correct PDA performed?

A

1 year

101
Q

When does ASD require treatment?

A

if large enough to cause right ventricle dilation

102
Q

What is the management of large secundum ASDs?

A

cardiac catheterisation with insertion of an occlusion device

103
Q

What is the management of large AVSD?

A

surgical correction

104
Q

At what age is catheter device closure for secundum ASD performed?

A

3-5 years

105
Q

At what age is surgery for partial AVSD performed?

A

3 years of age

106
Q

What is the typical presentation of right-to-left shunts?

A

cyanosis (blue, oxygen saturations ≤94%, or collapsed), usually in the first week of life

107
Q

What is the test used to determine the presence of heart disease in a cyanosed neonate?

A

Hyperoxia (nitrogen washout) test

108
Q

What does the hyperoxia (nitrogen washout) test involve to determine the presence of heart disease in a cyanosed neonate?

A

infant placed in 100% oxygen (headbox or ventilator) for 10 minutes

right radial arterial partial pressure of oxygen measured frmo blood gas

109
Q

How is the result from the hyperoxia (nitrogen washout) test interpreted?

A
  • if right radial arterial partial pressure of oxygen from blood gas remains low (<15kPa, 113 mmHg) after this time, diagnosis of cyanotic (right to left) heart disease can be made if lung disease and persistent pulmonary hypertension of newborn have been excluded
  • if PaO2 is over 20kPa, not cyanotic heart disease
110
Q

What are the 2 steps of management of the cyanosed neonate?

A
  • Stabilise ABC, with artificial ventilation if necessary
  • Start prostaglandin infusion (5 ng/ kg per min)
111
Q

Why is it important to give a prostaglandin infusion quickly for the cyanosed neonate?

A

most infants with cyanotic heart disease in first few days of life are duct dependent i.e. there is reduced mixing between pink oxygenated blood returning from lungs and blue deoxygenated blood from the body

Maintenance of ductal patency key to early survival of these children

112
Q

When giving prostaglandins for cyanotic neonates, what are 5 side effects to look out for?

A
  1. Apnoea
  2. Jitteriness and seizures
  3. Flushing
  4. Vasodilatation
  5. Hypotension
113
Q

What is the most common cause of cyanotic congenital heart disease?

A

Tetralogy of Fallot

114
Q

What are the 4 features of tetralogy of Fallot?

A
  1. Large VSD
  2. Overriding of the aorta with respect to the ventricular septum
  3. Subpulmonary stenosis causing right ventricular outflow tract obstruction
  4. Right ventricular hypertrophy as a result
115
Q

What are 2 ways that Tetralogy of Fallot may be diagnosed?

A
  1. Antenatally
  2. Following identification of murmur in the first 2 months of life
  3. Severe cyanosis in first few days of life
116
Q

What were the 3 features of the classical description of clinical features of Tetralogy of Fallot (no longer common now in developed countries)?

A
  1. Severe cyanosis
  2. Hypercyanotic spells
  3. Swuatting on exercise in late infancy
117
Q

What is the significance of hypercyanotic spells which might be associated with Tetralogy of Fallot?

A

may lead to myocardial infarction, cerebrovascular accidents and even death if left untreated

118
Q

What do hypercyanotic spells which may occur in Tetralogy of Fallot involve?

A

rapid increase in cyanosis, usually associated with irritability or inconsolable crying because of severe hypoxia and breathlessness and pallow because of tissue acidosis

short murmur during a spell on auscultation

119
Q

What are 2 physical signs of Tetralogy of Fallot?

A
  1. Clubbing of fingers and toes in older children
  2. Loud harsh ejection systolic murmur at left sternal edge from day 1 of life
120
Q

What will happen over time in Tetralogy of Fallot and why?

A

increasing right ventricular outflow tract obstruction, predominantly muscular and below the pulmonary valve, murmur will shorten and cyanosis will increase

121
Q

Why is Tetralogy of Fallot considered an example of a right to left shunt?

A

the right ventricular outflow tract obstruction due to subpulmonary stenosis results in blood flowing from right to left across the ventricular septal defect

122
Q

What will a chest radiograph show in tetralogy of Fallot? 3 features

A
  • small heart with uptilted apex (boot-shaped) due to right ventricular hypertrophy, more prominent in older child
  • may be right-sided aortic arch, but characteristically there’s a pulmonary artery ‘bay’, a concavity on the left heart border where the convex-shaped main pulmonary artery and right ventricular outflow tract would normally be profiled
  • decreased pulmonary vascular markings - reduced pulmonary blood flow
123
Q

What will ECG show in Tetralogy of Fallot?

A

normal at birth, right ventricular hypertrophy when older

124
Q

What will echocardiography show in Tetralogy of Fallot?

A

will demonstrate the cardinal features but cardiac catheterisation may be required to show the detailed anatomy of the coronary arteries

125
Q

What is the management of Tetralogy of Fallot? 3 aspects

A
  • initial management medical
  • definitive surgery around 6 months of age: closing VSD, relieving right ventricular outflow tract obstruction, sometimes with an artificial patch which extends across the pulmonary valve
  • if very cyanosed in neonatal period, require shunt to increase pulmonary blood flow - artificial tube from subclavian to pulmonary artery (Blalock-Taussig shunt) or balloon dilatation of right ventricular outflow tract
126
Q

At what age is definitive surgery for tetralogy of Fallot performed?

A

6 months

127
Q

What are the 2 types of surgery that may be performed for Tetralogy of Fallot in neonates who are very cyanosed?

A
  1. Shunt with artificial tube from subclavian artery to pulmonary artery (modified Blalock-Taussig shunt)
  2. Balloon dilatation of right ventricular ventricular outflow tract
128
Q

At what point should you treat the hypercyanotic episodes of Tetralogy of Fallot?

A

usually self-limiting and followed by long period of sleep

if prolonged (beyond 15 min) - give prompt treatment

129
Q

What are 5 aspects of the treatment of hypercyanotic episodes of Tetralogy of Fallot?

A
  1. Sedation and pain relief (morphine)
  2. IV propranolol (or alpha blocker)
  3. IV volume administration
  4. Bicarbonate to correct acidosis
  5. Muscle paralysis and artificial ventilation to reduce metabolic oxygen demand
130
Q

How do IV propranolol or an alpha-adrenoceptor agonists work to treat hypercyanotic episodes of Tetralogy of Fallot?

A

probably as peripheral vasoconstrictor and by relieving subpulmonary muscular obstruction that is the cause of reduced pulmonary blood flow

131
Q

What is transposition of the great arteries?

A
  • aorta connected to right ventricle and pulmonary artery connected to left ventricle (discordant ventriculo-arterial connection)
  • blue blood is returned to body and pink blood returned to lungs
  • 2 parallel circulations - unless there is mixing of blood between them
  • incompatible with life
132
Q

What are 4 ways that mixing of blood in transposition of the great arteries with 2 separate, parallel circulations can occur?

A
  1. VSD
  2. ASD
  3. PDA
  4. Therapeutic interventions to achieve mixing
133
Q

What is the usual presentation of transposition of the great arteries?

A
  • cyanosis - usually on day 2 of life when ductal closure leads to reduction in mixing of blood
134
Q

When does the cyanosis of transposition of the great arteries usually occur and why?

A

day 2 of life - closure of ductus arteriosus leads to reduction in mixing of desaturated and saturated blood

135
Q

What are 3 physical signs of transposition of the great arteries?

A
  1. Cyanosis - always
  2. Second heart sound often loud and single
  3. Usually no murmur but may be systolic murmur from increased flow or stenosis within the left ventricular (pulmonary) outflow tract
136
Q

What is likely to be seen on CXR in transposition of the greater arteries?

A

narrow upper mediastinum with ‘egg on side’ appearance of cardiac shadow (due to anteroposterior relationship of greater vessels, narrow vascular pedicle, and hypertrophied righ ventricle, respsectively)

increased pulmonary vascular markings are common due to increased pulmonary blood flow

137
Q

What are the usual ECG findings in transposition of the great arteries?

A

usually normal

138
Q

What will echo show in transposition of the great arteries?

A

abnormal arterial connections and associated abnormalities

139
Q

What is the immediate management of transposition of the great arteries?

A
  • maintain patency of ductus arteriosus with prostaglandin infusion is mandatory
  • 20% will need balloon atrial septostomy: catheter with inflatable balloon passed through umbilical or femoral vein, tears atrial septum to open foramen ovale
140
Q

What is the definitive management of transposition of the great arteries?

A

surgery: usually arterial switch procedure in neonatal period

pulmonary artery and aorta transected above arterial valves and switched

coronary arteries transferred across to new aorta

141
Q

When is surgery for transposition of the great arteries usually performed?

A

first few days of life

142
Q

What is the natural history of the development of Eisenmenger syndrome?

A
  • left-to-right shunt or common mixing not treated so pulmonary arteries become thick walled, resistance to flow increases
  • gradually become less symptomatic as shunt decreases
  • eventually at 10-15 years of age shunt reverses, teenager goes blue
143
Q

At what age does the shunt reverse in Eisenmenger syndrome and what happens as a result?

A

10-15 years of age

becomes cyanotic

144
Q

What is the outcome of Eisenmenger syndrome?

A

will die in right heart failure at variable age - usually 4th or 5th decade of life

145
Q

What is the treatment for Eisenmenger syndrome?

A

prevention: early intervention

heart-lung transplantation - not easily available

medicine available to palliate pulmonary vascular disease

146
Q

What are 2 types of common mixing conditions of the heart?

A
  1. Complete AVSD
  2. Complex congenital heart disease, e.g. tricuspid atresia
147
Q

When is atrioventricular septal defect (complete) most commonly seen?

A

Down syndrome

148
Q

What is complete AVSD?

A

defect in the middle of the heart with a single five-leaflet (common) valve between the atria and ventricles, which stretches across the entire atrioventricular junction and tends to leak

149
Q

What happens secondary to a complete AVSD?

A

pulmonary hypertension

150
Q

What are 4 of the key features of a complete AVSD?

A
  1. Presentaiton of antenatal ultrasound screening
  2. Cyanosis at birth or heart failure at 2-3 weeks of life
  3. No murmur heard, lesion being detected on routine echocardiography screening in a newborn baby with Down syndrome
  4. Always a superior axis on ECG
151
Q

What is always seen in complete AVSD on the ECG?

A

superior axis

152
Q

What is the management of complete AVSD?

A
  • treat heart failure medically as for large VSD (diuretics, captopril, calories)
  • Surgical repair at 3-6 months of age
153
Q

What is the time of surgical management of complete AVSD?

A

3 to 6 months of age

154
Q

What are 4 examples of complex congenital heart disease that can cause mixing (blue and breathless)?

A
  1. Tricuspid atresia (most common)
  2. Mitral atresia
  3. Double inlet left ventricle
  4. Common arterial trunk - truncus arteriosus
155
Q

What is tricuspid atresia?

A

only the left ventricle is effective, the right is small and non-functional, due to absence of tricuspid valve

leads to common mixing of systemic and pulmonary venous return in the left atrium

156
Q

What is the typical presentation of tricuspid atresia?

A

cyanosis in the newborn period if duct dependent, or may be well at birth and become cyanosed or breathless

157
Q

What are 2 aspects of the management of tricuspid atresia?

A
  1. Blalock-Taussig shunt insertion (between subclavian and pulmonary arteries)
  2. Pulmonary artery banding operation to reduce pulmonary blood flow if breathless
158
Q

Is completely corrective surgery possible for tricuspid atresia and why?

A

not usually - only one effective functinoing venricle

159
Q

What palliative surgery can be performed later in the life of a patient with tricuspid atresia? 2 forms

A
  • Glenn or hemi-Fontan operation connecting superior vena cava to the pulmonary artery after 6 months of age
  • Fontan operation to connet inferior vena cava to pulmonary artery at 3-5 years
160
Q

What is the method of the palliative surgery later in the life of patients with tricuspid atresia?

A

left ventricle drives blood around the body and systemic venous pressure supplied blood to the lungs

relieves cyanosis, removes long-term volume load on single functional ventricle

161
Q

What are 3 types of outflow obstruction in the well child?

A
  1. Aortic stenosis
  2. Pulmonary stenosis
  3. Adult-type coarctation of the aorta
162
Q

What causes aortic stenosis in a child?

A

aortic valve leaflets are partly fused together, giving a restrictive exit from the left ventricle

may be one to three aortic leaflets

163
Q

What are 2 things that may co-exist with aortic stenosis that should be excluded?

A
  1. Mitral valve stenosis
  2. Coarctation of the aorta
164
Q

How does the majority of aortic stenosis in children present?

A

asymptomatic murmur

165
Q

How may patients with critical aortic stenosis present?

A

in neonatal period, if duct-dependent systemic circulation: may present with severe heart failure leading to shock

166
Q

What are 5 physical signs of aortic stenosis?

A
  1. Small volume, slow rising pulses
  2. Carotid thrill (always)
  3. Ejection systolic murmur maximal at upper right sternal edge radiating to the neck
  4. Delayed and soft aortic second sound
  5. Apical ejection click
167
Q

What will be seen on CXR in aortic stenosis?

A

normal or prominent left ventricle with post-stenotic dilatation of the ascending aorta

168
Q

What may be seen on the ECG in aortic stenosis?

A

left ventricular hypertrophy

169
Q

How do you decide when to intervene with aortic stenosis in children?

A

regular clinical and echocardiographic assessment

if symptoms on exercise or high resting pressure gradient (>64 mmHg) across aortic valve will undergo balloon valvotomy

170
Q

What is the management of aortic stenosis, when indicated?

A
  • balloon valvotomy - balloon dilatation to open valve
  • most requiring treatment in first few years will eventually require aortic valve replacement
171
Q

In which age group is balloon valvotomy to treat aortic stenosis dangerous?

A

in neonates - generally safe in older children

172
Q

What is pulmonary stenosis?

A

pulmonary valve leaflets are fused together, giving restrictive exist from the right ventricle

173
Q

What is the usual presentation of pulmonary stenosis?

A

most asymptomatic, diagnosed clinically

174
Q

How might pulmonary stenosis present if critical?

A

in neonates, have duct-dependent pulmonary circulation and present in first few days of life with cyanosis

175
Q

What are 3 physical signs of pulmonary stenosis?

A
  1. Ejection systolic murmur, best heart upper left sternal edge; thrill may be present
  2. Ejection click best heard at upper left sternal edge
  3. If severe, prominent right ventricular impulse (heave)
176
Q

What will CXR show in pulmonary stenosis?

A

normal or post-stenotic dilatation of the pulmonary artery

177
Q

What will ECG show in pulmonary stenosis?

A

evidence of right ventricular hypertrophy (upright T wave in V1)

178
Q

When is intervention requirement for pulmonary stenosis?

A

when pressure gradient across pulmonary valve becomes markedly increased (>64mmHg)

179
Q

What is the treatment of choice for pulmonary stenosis?

A

transcatheter balloon dilatation

180
Q

What is the natural course of adult-type coarctation of the aorta?

A

gradually becomes severe over many years

181
Q

What are 5 possible physical signs of adult-type coarctation of the aorta?

A
  1. Asymptomatic
  2. Systemic hypertension in the right arm
  3. Ejection systolic murmur at upper sternal edge
  4. Collaterals heard with continuous murmur at the back
  5. Radio-femoral delay.
182
Q

What causes radio-femoral delay in adult-type coarctation of the aorta?

A

blood bypassing the obstruction via collateral vessels in the chest wall and hence pulse in legs is delayed

183
Q

What will be seen in CXR in adult-type coarctation of the aorta?

A
  • ‘rib notching’ due to the development of large collateral intercostal arteries running under the rubs posteriorly to bypass the obstruction
  • ‘3’ sign, with visible notch in the descending aorta at the site of the coarctation
184
Q

What is seen on the ECG in adult-type coarctation of the aorta?

A

left ventricular hypertrophy

185
Q

What is the management of of adult-type aortic stenosis?

A

when becomes severe (on echo), insert stent at cardiac catheter

sometimes surgical repair required

186
Q

What are 3 causes of outflow obstruction in the sick infant?

A
  1. Coarctation of the aorta
  2. Interruption of the aortic arch
  3. Hypoplastic left heart syndrome
187
Q

What is the presentation of outflow obstruction in a sick infant?

A

heart failure and shock in neonatal period, unless diagnosed on antenatal ultrasound

188
Q

When do children who are sick with outflow obstruction present?

A

in the neonatal period (unless diagnosed on antenatal ultrasound)

189
Q

What is the management of outflow obstruction in the sick child? 3 aspects

A
  1. ABC
  2. Prostaglandin ASAP
  3. Referral to cardiac centre for early surgical intervention
190
Q

What is coarctation of the aorta?

A

due to arterial duct tissue encircling the aorta just at the point of insertion of the duct - when duct closes, aorta also constricts, causing severe obstruction to left ventricular outflow

191
Q

How do neonates often present with coarctation of the aorta?

A

acute circulatory collapse at 2 days of age when duct closes

192
Q

What are 3 physical signs of coarctation of the aorta?

A
  1. Sick baby, with severe heart failure
  2. Absent femoral pulses
  3. Severe metabolic acidosis
193
Q

What with a CXR show in coarctation of the aorta?

A

cardiomegaly from heart failure and shock

194
Q

What will an ECG show in coarctation of the aorta?

A

normal

195
Q

What is the management of coarctation of the aorta?

A

ABC, prostaglandins ASAP, surgical repair soon after diagnosis

196
Q

What is interruption of the aortic arch?

A

no connection between proximal aorta and distal to the arterial duct, so cardiac output dependent on right-to-left shunt via the duct

197
Q

What is usually present in association with interruption of the aortic arch?

A

VSD

198
Q

What is the presentation of interruption of the aortic arch?

A

shock in the neonatal period

199
Q

What is the management of interruption of the aortic arch?

A

complete correction with closure of the VSD and repair of the aortic arch usually performed within the first few days of life

200
Q

What syndrome can interruption of the aortic arch be a part of? What are 5 aspects of this?

A

DiGeorge syndrome

cleft palate/palatal defects, abnormal immunity (immunodeficiency) Thymic aplasia, cardiac anomalies, hypoclacaemia, 22q11.2 microdeletion

201
Q

What happens in hypoplastic left heart syndrome?

A

underdevelopment of the entire left side of the heart. mitral valve small or atretic, left ventricle is diminutive, there is usually aortic valve atresia

ascending aorta small, and almost invariably coarctation of the aorta

202
Q

What are 2 ways that hypoplastic left heart may present?

A
  1. Antenatal ultrasound screening
  2. Extremely sick neonate, duct-dependent systemic circulation - profound acidosis, rapid cardiovascular collapse
203
Q

What are 3 physical features of hypoplastic left heart?

A
  1. Profound acidosis
  2. Rapid cardiovascular collapse
  3. Weakness or absence of all peripheral pulses (vs waek femoral pulses in coarctation of aorta)
204
Q

What is the management of hypoplastic left heart syndrome?

A
  • Norwood procedure: neonatal operation
  • if SGA or complex lesions, hybrid procedures with cardiac catheter + surgical operation
  • Followed by Glenn or Hemi-Fontan at about 6 months of age and again (Fontan) at about 3 years of age

Paeds: Acute Presentation (14 decks)

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